Treating juvenile dermatomyositis to target: Paediatric Rheumatology European Society/Childhood Arthritis and Rheumatology Research Alliance-endorsed recommendations from an international task force.

Objectives: Despite the recent prognostic improvement, a sizeable proportion of patients with juvenile dermatomyositis (JDM) respond suboptimally to contemporary therapies. This study aimed to develop recommendations for treating JDM to target.

Methods: A Steering Committee formulated a set of provisional recommendations based on evidence derived from a systematic literature review and a retrospective chart review of patients.

Correlation Between Interferon Response Gene Score and Disease Activity in Juvenile Dermatomyositis.

Objective: Type 1 interferons (IFNs) have been identified as potentially important measures of disease activity in juvenile dermatomyositis (JDM). An IFN response gene (IRG) score has been defined using NanoString technology and appears to correlate with disease activity in cross-sectional samples of patients with JDM. This study aimed to determine if there is evidence of a correlation between disease activity and IRG score in patients with JDM, both early in the disease course and longitudinally.

Early Bilateral Gonadoblastoma in a Young Child with Mosaicism for Turner Syndrome and Trisomy 18 with Y Chromosome.

Mosaic Turner syndrome (TSM) commonly occurs in the form of 45,X/46,XX and 45,X/46,X,i(X)(q10). Mosaicism for a Y chromosome, 45,X/46,XY, has been well documented and is associated with increased risk of gonadoblastoma (GB). To date, there are only six reported cases of TSM with a trisomy 18 karyotype, and only two of these were phenotypically female with 45,X/47,XY,+18 karyotype.